Early exfoliation of permanent tooth in patient with hypophosphatasia
Early exfoliation of permanent tooth in patient with hypophosphatasia
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DOI：
10.1016/j.pdj.2017.08.005
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发表时间：
2017-12-01
影响因子：
0.8
通讯作者：
Nakano, Kazuhiko
中科院分区：
文献类型：
--
作者：
Okawa, Rena;Miura, Jiro;Nakano, Kazuhiko
背景资料：低磷酸酶症（HPP）是一种罕见的遗传性骨骼疾病，由编码组织非特异性碱性磷酸酶的ALPL基因突变引起，由于牙骨质形成紊乱而导致乳牙早期脱落，通常被认为是主要的牙齿表现。病例报告：一名11岁的男孩被诊断为儿童型HPP被转介到我们医院的上颌右中切牙脱落。患牙的微型计算机断层扫描结果显示外根吸收，釉质发育不全，牙本质薄，髓室宽，而牙骨质形成紊乱，釉质发育不全，牙本质矿化不足，釉质交界处周围牙骨质不足。恒牙可能会有早期脱落的风险，以及其他结构异常的HPP患者，因此，受影响的患者需要纵向牙科随访检查。(c)2017年日本儿童牙科学会。由爱思唯尔有限公司出版。保留所有权利。
Background: Hypophosphatasia (HPP) is a rare inherited skeletal disorder caused by mutations in the ALPL gene encoding tissue-nonspecific alkaline phosphatase, with early exfoliation of primary teeth due to disturbed formation of cementum often recognized as a major dental manifestation. However, reports regarding permanent teeth in HPP cases are scant.Case report: An 11-year-old boy diagnosed with childhood type HPP was referred to our hospital for exfoliation of the maxillary right central incisor. Micro-computed tomography findings of the affected tooth revealed external root resorption, enamel hypoplasia, thin dentin, and a wide pulp chamber, while disturbed cementum formation, enamel hypoplasia, dentin hypo-mineralization, and scant cementum around the enamel junction were observed by scanning electron microscopy.Conclusion: Permanent teeth may have a risk of early exfoliation as well as other structural abnormalities in HPP patients, thus longitudinal dental follow-up examinations of affected patients are required. (c) 2017 Japanese Society of Pediatric Dentistry. Published by Elsevier Ltd. All rights reserved.